friday, december 5 th, 2008. the patient was appropriately resuscitated with crystalloid fluid and...
TRANSCRIPT
Friday, December 5th, 2008
The patient was appropriately resuscitated with crystalloid fluid and blood products
Emergent endoscopy showed large gastric and esophageal varices with stigmata of recent bleeding. No endoscopic therapies or biopsies were performed at the time.
Once stable, a three-phase abdominal CT with IV contrast was performed. A diagnostic test/procedure was then performed.
Dr. Emma Robinson
Dr. Gerald VillanuevaDepartment of Medicine
Division of Gastroenterology
Dr. Sameer Dhalla
Stool Culture: Negative Fecal Leukocytes: Negative Stool Ova and Parasites: Negative Hepatitis Serologies: Negative ANA, AMA: Negative Ceruloplasmin, anti-trypsin: WNL Tests for Thrombophilia: All Negative Anti-Schistosomal Antibodies: Negative
A diagnostic liver biopsy was performed
Dr. Cristina Hajdu
Findings MINIMAL PORTAL AND LOBULAR INFLAMMATION
FOCAL PORTAL, PERIPORTAL AND PERICENTRAL VEIN FIBROSIS
MINIMAL MACROVESICULAR STEATOSIS
Final Diagnosis
Idiopathic Portal Fibrosis
Idiopathic Portal Fibrosis
Young previously healthy man from Hong Kong with short history of heavy alcohol use presents with UGIB and hypovolemia
Anemia and Hypoalbuminemia Clinical and radiographic evidence of
portal hypertension: variceal bleed, ascites, Splenomegaly. All out of proportion to mild hepatocellular disease
No cirrhosis on CT. No venous thrombosis
PrehepaticPortal vein thrombosis
Splenic vein thombosis
Splanchnic arteriovenous fistula
Splenomegaly (lymphoma, Gaucher's disease)
PosthepaticIVC obstruction
Cardiac disease (constrictive pericarditis, restrictive cardiomyopathy)
Intrahepatic
PresinusoidalSchistosomiasisIdiopathic portal hypertension/Noncirrhotic portal fibrosis/Hepatoportal sclerosis
Primary biliary cirrhosisSarcoidosisCongenital hepatic fibrosisSclerosing cholangitisHepatic arteriopetal fistula
SinusoidalArsenic poisoningVinyl chloride toxicityVitamin A toxicityNodular regenerative hyperplasia
PostsinusoidalSinusoidal obstruction syndrome (Veno-occlusive disease)Budd-Chiari syndrome
PrehepaticPortal vein thrombosis
Splenic vein thombosis
Splanchnic arteriovenous fistula
Splenomegaly (lymphoma, Gaucher's disease)
PosthepaticIVC obstruction
Cardiac disease (constrictive pericarditis, restrictive cardiomyopathy)
Intrahepatic
PresinusoidalSchistosomiasisIdiopathic portal hypertension/Noncirrhotic portal fibrosis/Hepatoportal sclerosis
Primary biliary cirrhosisSarcoidosisCongenital hepatic fibrosisSclerosing cholangitisHepatic arteriopetal fistula
SinusoidalArsenic poisoningVinyl chloride toxicityVitamin A toxicityNodular regenerative hyperplasia
PostsinusoidalSinusoidal obstruction syndrome (Veno-occlusive disease)Budd-Chiari syndrome
Historical 19th century term was Banti’s Syndrome: Anemia, thrombocytopenia, splenomegaly without hematological cause
Characterized simultaneuosly in the 1960’s-India (1962): Non-Cirrhotic Portal Fibrosis-Japan (1962): Idiopathic Portal hypertension-US (1965): Hepatoportal Sclerosis
After 30 years of competing names for the same disease, the above term has been “generally” adopted
Presence of portal hypertension Absence of liver cirrhosis Histological features of dense portal
fibrosis, marked phlebosclerosis, and dilated sinusoids.
Present worldwide but most focused in South Asia and East Asia, particularly Japan
Prevalence: 25-30% of non-cirrhotic portal hypertension in Asia. Dramatic decline in a more recent Japanese population survey.
Disparate Male to Female Ratios
Recurrent Infection Autoimmunity Genetic: HLA-DR3 Hypercoagulability HAART Miscellaneous Toxins
Variceal Bleed which is surprisingly well tolerated
Other signs of portal hypertension Preserved Liver Function Characteristic Hemodynamics Characteristic Path Findings
Diagnosis of exclusion
Histological feature*Frequency,
percent
Irregular intimal thickening of portal veins 75-100
Organizing thrombus and/or recanalization of portal veins
20-100
Intralobular fibrous septa 95
Abnormal blood vessels in the lobules 75
Subcapsular atrophy 70
Dense portal fibrosis and portal venous obliteration 32-52
Periductal fibrosis of interlobular bile ducts 50
Portal inflammation 47
Nodular hyperplasia of parenchyma 25-40
Few studies of IPF management exist Acute and Prophylactic regimens for
variceal bleed as with cirrhotics TIPS and surgical anastomosis is
often well tolerated
Small subgroup progress to nodular transformation of the liver with extensive subhepatic and portal fibrosis
HCC?
The Patient is doing well on his previous regimen of nadolol and esomeprazole
Furosemide and Aldactone were added for ascites
He is following regularly with a gastroenterologist and has had no recurrent bleeding events since his discharge in October 2008
Idiopathic Portal Fibrosis
Portal Hypertension
Alcohol Abuse
Asian Descent
Raised in Endemic Area
UnknownMechanisms
Gastric/Esophageal Varices complicated by recurrent UGIB
Multifactorial Anemia
OrthostasisSplenomegaly
Mild Elevation in Alk Phos and ALT
Steatosis and Mild peri-central vein fibrosis
Medication non-adherence
Ascites
Dr. Martin Blaser Dr. Anthony Grieco Dr. Emma Robinson Dr. Gerald Villanueva Dr. Cristina Hajdu Dr. Chirayu Gor Dr. Christina Yoon